Abstract A 19 year old healthy female with a history of iron deficiency anemia presented with progressively worsening non-massive hemoptysis over the past 8 months, since patient contracted COVID-19. CT angiography showed a focal AVM with tortuosity of vasculature in the superior segment of the right lower lobe and in the mediastinum. IR was unable to embolize due to complex anatomy, and patient was transferred to our facility for further workup. Further review of imaging suggested that the vascular malformation was a veno-venous malformation, and it was noted that the left pulmonary veins were obliterated. VQ scan confirmed near-complete loss of perfusion to the left lung. Patient underwent bronchoscopy with biopsy showing severe mucosal capillary dilation with mild hemorrhage in the left mainstem bronchus and right middle lobe, and stigmata of bleeding throughout the entire left mainstem bronchus concerning for pulmonary hypertension. Biopsy of lymph node station 7 was negative for malignancy and did not show any granulomatous inflammation. Right heart catheterization confirmed precapillary pulmonary hypertension with mPAP of 29, PVR of 4.8 Wood units, and normal pulmonary capillary wedge pressure. Bubble study was negative, and there was no evidence of left to right shunting. Autoimmune workup showed elevated MPO levels with no other abnormal antibody levels or inflammatory markers. It is hypothesized that mediastinal fibrosis, led to pulmonary vein obliteration increasing pulmonary capillary membrane pressures. These pressures resulted in sequelae of pulmonary hypertension which manifested as capillary dilatation and subsequent hemoptysis. Mediastinal fibrosis is a rare, benign disorder in which progressive and dense fibrosis of the mediastinum can lead to encasement or invasion of the mediastinal structures. The most common initial symptoms are cough, dyspnea, and recurrent pneumonia. Hemoptysis is often reported, however not as an initial symptom. The specific etiology of the condition is rare, however most cases are infectious in etiology secondary to tuberculosis, histoplasmosis, sarcoidosis, silicosis or other fungal diseases. Diagnosis is based on clinical presentation and exclusion of malignancy, in addition to workup of autoimmune causes such as IgG4 related disease. Unfortunately, there is no focused therapy for fibrosing mediastinitis; treatment is focused on symptomatic relief and management of underlying complications. This case highlights the importance of clinician image review, and broad differential diagnosis when dealing with hemoptysis. She requires further infectious and autoimmune workup to delineate the underlying cause of her pulmonary fibrosis. Multidisciplinary planning regarding possible recanalization options for her pulmonary veins is ongoing. This abstract is funded by: none
Zahedi et al. (Fri,) studied this question.